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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Table 2.

Cumulative Risk of Cancers in Peutz-Jeghers Syndrome

Cancer SiteGeneral Population RiskPeutz-Jeghers Syndrome
RiskMean Age at Diagnosis
Colorectal5%39%42-46 yrs
Stomach<1%29%30-40 yrs
Small bowel<1%13%37-42 yrs
Breast12.4%32%-54%37-59 yrs
Ovarian (mostly SCTAT)1.6%21%28 yrs
Cervix (adenoma malignum)<1%10%34-40 yrs
Uterine2.7%9%43 yrs
Pancreas1.5%11%-36%41-52 yrs
Testicular (Sertoli cell tumor)<1%9%6-9 yrs
Lung6.9%7%-17%47 yrs

Adapted from Syngal et al [2015]

SCTAT = sex cord tumor with annular tubules

From: Peutz-Jeghers Syndrome

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